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Subacute sclerosing panencephalitis: A clinical appraisal

Sujit Abajirao Jagtap1, M D Nair, Harsha J Kambale

  • 1Department of Neurology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum, Kerala, India.

Annals of Indian Academy of Neurology
|December 17, 2013
PubMed
Abstract

Insights

Subacute sclerosing panencephalitis (SSPE) predominantly affects children and young adults, often linked to low socioeconomic status. Adult-onset SSPE shares clinical features with childhood cases, with a longer measles-to-symptom interval observed.

Area of Science:

  • Neurology
  • Infectious Diseases
  • Pediatrics

Background:

  • Subacute sclerosing panencephalitis (SSPE) is a rare, progressive neurological disorder.
  • It results from persistent measles virus infection in immune-resistant individuals.
  • SSPE primarily impacts children and young adults.

Purpose of the Study:

  • To delineate the clinical characteristics and natural progression of SSPE.
  • To analyze demographic, clinical, electrophysiological, and imaging findings in SSPE patients.

Main Methods:

  • Data collected from 34 SSPE patients diagnosed between 2004-2010 using Dyken's criteria.
  • Analysis included demographical, clinical, electrophysiological (EEG), and neuroimaging (MRI) features.
  • Cerebrospinal fluid (CSF) anti-measles antibody levels were assessed.

Main Results:

  • The study included 34 patients (76.5% male), with onset ages ranging from 3 to 31 years.
  • Childhood SSPE (n=21) and adult-onset SSPE (n=13) were observed; 85.3% had low socioeconomic status.
  • Common symptoms included scholastic backwardness (52.5%) and seizures (23.5%). EEG showed characteristic periodic complexes, and all patients had positive CSF anti-measles antibodies. Mean incubation period was 9.6 years.

Conclusions:

  • SSPE is more prevalent in individuals from low socioeconomic backgrounds.
  • Adult-onset SSPE mirrors childhood SSPE clinically, with a notable exception of a longer interval from measles infection to ophthalmic symptoms presentation.
  • The study highlights the long-term, progressive nature of SSPE, with varied patient outcomes during follow-up.
Keywords:
Measlesmyoclonussubacute sclerosing panencephalitis

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